Beghetti M, Berner M, Rimensberger P C
Cardiology Unit, Hôpital des Enfants, Department of Paediatrics, University Hospital of Geneva, 1211 Genève 14, Switzerland.
Heart. 2001 Sep;86(3):E10. doi: 10.1136/heart.86.3.e10.
Primary pulmonary hypertension is a rare disease in childhood associated with a poor prognosis. However, during the past 10 years, pulmonary vasodilator treatment has somewhat improved its prognosis. Long term continuous infusion of prostacyclin (epoprostenol) has been shown to improve physical capacity and to reduce mortality in primary and secondary pulmonary hypertension. It has been reported in adults that daily repetitive inhalation of iloprost, a prostacyclin analogue, seems also suitable for long term therapy of pulmonary hypertension. Repetitive inhalation of iloprost was administered to a 5 year old boy with severe primary pulmonary hypertension. He showed continuous clinical improvement without any side effects over the three years of treatment. This treatment may offer an alternative to continuous intravenous prostacyclin infusion and obviates the need for a permanent central venous catheter.
原发性肺动脉高压是一种儿童期罕见疾病，预后较差。然而，在过去10年中，肺血管扩张剂治疗在一定程度上改善了其预后。长期持续输注前列环素（依前列醇）已被证明可改善原发性和继发性肺动脉高压患者的体能并降低死亡率。据报道，在成人中，每日重复吸入前列环素类似物伊洛前列素似乎也适用于肺动脉高压的长期治疗。一名患有严重原发性肺动脉高压的5岁男孩接受了伊洛前列素重复吸入治疗。在三年的治疗过程中，他的临床症状持续改善且无任何副作用。这种治疗方法可能是持续静脉输注前列环素的一种替代方案，并且无需长期留置中心静脉导管。
